Berinert Prevents Acute Swelling in HAE Patient After Oral Surgery

Patricia Inácio, PhD avatar

by Patricia Inácio, PhD |

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Short-term treatment with Berinert, a plasma-derived form of the C1 inhibitor protein, successfully prevented acute swelling attacks in a patient with hereditary angioedema (HAE) following oral surgery, a case report describes.

The report, “Oral Surgery Procedures in a Patient Affected by Hereditary Angioedema Type I,” was published in the journal Case Reports in Dentistry.

HAE type 1 is a rare genetic disease caused by a deficiency in the production of C1 inhibitor (C1-INH), a protein that controls the activity of other proteins involved in inflammation. The disease also is characterized by low levels of C4, a component of the complement cascade, which is part of the immune system.

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People with HAE can experience sudden episodes of swelling in the deeper layers of the skin, including on the face. These acute attacks are generally unpredictable, but can be triggered by stress, as well as dental treatment and oral surgeries.

This report describes the case of a patient with HAE type 1 who was referred to oral surgery after undergoing short-term preventive (prophylactic) treatment with a C1-INH concentrate.

The patient, a 37-year-old man, was admitted to the oral surgery department of the University-Hospital of Pisa in Italy, after experiencing swelling episodes in the area below his lower incisors. During examination, clinicians noticed a mild swelling in his lower jaw, specifically below the incisors.

To investigate the origin of the swelling, an X-ray was performed. The test revealed an area of radiolucency, or near transparency — indicative of bone tissue destruction — around the patient’s incisors.

Moreover, clinicians found certain teeth were negative in the pulp vitality test, which measures the presence of pulp blood flow — a measure of tooth health — and others were sensitive to percussion. Also, certain teeth were motile, meaning they were at risk of extraction.

The patient was diagnosed with an inflammatory odontogenic cyst and scheduled for surgery to remove the bone lesion and further evaluate the involved teeth.

Since he was 14, the patient experienced four episodes of swelling per year, affecting his hands, feet, face, and genitals. The swelling occurred slowly and only on a single area at a time and was always unaccompanied by fever.

The patient had been admitted to hospital in the past due to episodes of abdominal pain and diarrhea. Doctors suspected the patient had HAE, so he underwent blood tests which confirmed the diagnosis of HAE type 1. His C1-INH levels were below normal (7.8 mg/dl; normal range 12–30 mg/dl), as were his C4 levels (7.7 mg/dl; normal range 10–40 mg/dl).

His brother, who also had HAE type 1, died from asphyxia due to an HAE attack, which occurred after a dental extraction.

A comprehensive blood analysis, requested prior to surgery, was deemed normal. The patient was prescribed Berinert, which was slowly given into the vein at a dose of 1,500 international units for one hour before his surgery.

Marketed by CSL Behring, Berinert is made of a C1-INH concentrate derived from healthy donors. It is often used as a short-term preventive treatment for HAE attacks before surgery.

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The cyst was removed during surgery, as were three mandibular incisors, since they had marked bone loss and high mobility. A local anesthetic, called ropivacaine, was used in the surgical wound to lessen pain.

The patient scored his post-operative pain as a six, according to the Numeric Rating Score, which ranges from zero (no pain at all) to 10 (worst imaginable pain). He received treatment with an opioid pain medication, tramadol, given at a dose of 100 mg directly into the vein. A post-surgical antibiotic was also prescribed for seven days.

After being in the hospital for 36 hours for monitoring, the patient showed no signs of an HAE attack so was discharged. He was given a series of post-surgical instructions, including to go to the emergency room upon any suspicion of an attack.

He was also instructed on how to self-administer Firazyr (icatibant), an approved on-demand medicine to ease painful and potentially severe swelling attacks.

When he was reexamined seven days after surgery, no swelling had been reported. The patient had no pain, impairment of his mouth opening, or pus. There were also no signs of inflammation.

Nearly a month (28 days) after surgery, the patient showed good healing and excellent oral hygiene.

Overall, this case report shows that “a presurgical prophylaxis [preventive treatment] with C1- INH concentrate was effective in preventing acute HAE attacks and the postsurgical period was uneventful,” the researchers wrote.